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Amegakaryocytic thrombocytopenia with a positive direct Coombs' test.

A 50-year-old previously well woman developed a sudden onset of ecchymoses and petechiae over the trunk and extremities and gum bleeding. There was no history of alcohol use or drug ingestion. Physical examination was normal except for the ecchymoses, purpuric rash, and gum bleeding. Complete blood count showed Hb 8.0 g/dl, leucocytes 10.9 x 10(9)/L with a normal differential, platelets 8 x 10(9)/L and reticulocytes, 4%. The bone marrow was normocellular and showed absence of megakaryocytes; there was normoblastic erythroid hyperplasia and the myelopoiesis was normal. The direct Coombs test result was positive. There was poor response to treatment with prednisone and bolus doses of vincristine. Therapy with danazol resulted in the complete normalization of the blood counts, and the patient remains well 32 months after diagnosis and is currently taking 100 mg danazol daily.

Administration, Oral↗

[The 5q- syndrome associated with marked erythroid hypoplasia and Coombs test positive hemolysis].

The 5q- syndrome is a myelodysplastic disorder characterized by macrocytic anemia, hypolobulated micromegakaryocytic hyperplasia, and an interstitial deletion of chromosome 5 as a solitary cytogenetic abnormality. The majority of patients with this syndrome are elderly women exhibiting red blood cell transfusion-dependent refractory anemia with a normal-to-increased number of platelets and modest granulocytopenia. The prognosis is relatively favorable with a low incidence of leukemic transformation. We report on a patient with 5q- syndrome associated with autoimmune hemolytic anemia (AHIA) and severe erythroid hypoplasia mimicking pure red cell aplasia (PRCA). A 65-year-old woman was admitted because of severe anemia. Elevated serum levels of LDH and indirect bilirubin, and a positive direct Coombs' test suggested AIHA associated with a huge ovarian dermoid cyst. However, lack of peripheral reticulocytes and bone marrow eryhroblasts, characteristic megakaryocytic morphology, and solitary 5q- anomaly favored a diagnosis of 5q- syndrome complicated by PRCA-like feature. Underlying immunological abnormalities ascribed to aberrant lymphoid clones intrinsic to MDS may be responsible for red cell aplasia and autoantibodies against red blood cells.

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